Atypical teratoid rhabdoid tumor (AT/RT) is a rare tumor usually diagnosed in childhood. Although usually a brain tumor, AT/RT can occur anywhere in the central nervous system (CNS) including the spinal cord. About 60% will be in the posterior cranial fossa (particularly the cerebellum). One review estimated 52% posterior fossa, 39% sPNET (supratentorial primitive neuroectodermal tumors), 5% pineal, 2% spinal, and 2% multi-focal.
In the United States, three children per 1,000,000 or around 30 new AT/RT cases are diagnosed each year. AT/RT represents around 3% of pediatric cancers of the CNS. Around 17% of all pediatric cancers involve the CNS; it is the most common childhood solid tumor. The survival rate for CNS tumors is around 60%; with AT/RT it is around 10%. Pediatric brain cancer is the second leading cause of childhood death, just after leukemia. Recent trends suggest that the rate of overall CNS tumor diagnosis is increasing by about 2.7% per year. As diagnostic techniques using genetic markers improve and are used more often, the proportion of AT/RT diagnoses is expected to increase.
Because it is highly malignant, AT/RT has a high mortality rate. A survey of 36 AT/RT patients at St. Jude Children's Research Hospital from 1984 to 2003 showed that the two-year event-free survival for children under three was 11%, the overall survival rate was 17%. For children aged 3 years or older the event-free survival was 78% and the overall survival 89%. Because most patients with AT/RT are less than three years old (71% in St Jude Study), the overall prognosis for AT/RT is very poor.
Current research is focusing on using chemotherapy protocols that are effective against rhabdomyosarcoma in combination with surgery and radiation therapy.
Other articles related to "atypical teratoid rhabdoid tumor, atypical teratoid, tumor, rhabdoid tumor":
... Atypical teratoid/rhabdoid tumor was first described as a distinct entity in 1987 ... Before 1978, when rhabdoid tumor was described, AT/RT likely was misdiagnosed as medulloblastoma ... In some early reports the tumor was known also as malignant rhabdoid tumor (MRT) of the CNS ...
... In August 2011, a six-year-old named Avalanna Routh who was battling AT/RT at Dana–Farber Cancer Institute was given a pretend wedding with her idol Justin Bieber, with doctors and nurses providing a cardboard life-sized cutout of Bieber, a guitarist, flowers, and a T-shirt emblazoned with the words "Future Mrs ... Bieber" ...